Sturge-Weber Syndrome (encephalotrigeminal angiomatosis)

Symptoms:
• Port wine stains of the face
• Glaucoma/exopthalmos
• Seizures
• Mental retardation
• Obesity
• Leptomeningeal angioma
• Convulsions and hypotonia on contralateral side of birthmark
Etiology:
• Sporadic with no hereditary tendency abnormal arteriovenous formation
• 1 in 14K births
• Due to overabundance of capillaries around the ophthalmic branch of the trigeminal nerve
• Retardation due blood vessel malformation in pia mater leading to calcification
Diagnosis:
• Usually diagnosed at birth with port wine stains on the forehead and upper eyelid of one eye and seizures.
• Treatment is symptomatic – laser treatment of stains and medications for the seizures
Dental Concerns:
• Medications to control seizures can cause gingival enlargement in addition to fibrous enlargement directly related to the disease
• Obesity can contribute to periodontal disease
• In many cases, altered eruption patterns and teeth resorbed by the pathologic capillary nexus growth can result in oligodontia.
• Intraoral angiomas
Recent Info:
• The size of the facial lesions have no correlation with the severity of the brain lesion
o Can J Neurol Sci. 2008 Jul;35(3):301-7
• Severity of seizures in early childhood and their surgical correction directly correlate to the extent of mental retardation in adulthood
o Dev Med Child Neurol. 2000 Nov;42(11):756-9.